Narcolepsy is a rare brain disorder that reflects a selective loss or dysfunction of orexin (also known as hypocretin) neurons of the lateral hypothalamus. Narcolepsy type 1 (NT1) is characterized by excessive daytime sleepiness and cataplexy, accompanied by sleep-wake symptoms, such as hallucinations, sleep paralysis and disturbed sleep. Diagnosis is based on these clinical features and supported by biomarkers: evidence of rapid eye movement sleep periods soon after sleep onset; cerebrospinal fluid orexin deficiency; and positivity for HLA-DQB1*06:02. Symptomatic treatment with stimulant and anticataplectic drugs is usually efficacious. This Review focuses on our current understanding of how genetic, environmental and immune-related factors contribute to a prominent (but not isolated) orexin signalling deficiency in patients with NT1. Data supporting the view of NT1 as a hypothalamic disorder affecting not only sleep-wake but also motor, psychiatric, emotional, cognitive, metabolic and autonomic functions are presented, along with uncertainties concerning the 'narcoleptic borderland', including narcolepsy type 2 (NT2). The limitations of current diagnostic criteria for narcolepsy are discussed, and a possible new classification system incorporating the borderland conditions is presented. Finally, advances and obstacles in the symptomatic and causal treatment of narcolepsy are reviewed.

Narcolepsy - clinical spectrum, aetiopathophysiology, diagnosis and treatment.

Sleep disorders are frequent (18%–23%) and constitute a major risk factor for psychiatric, cardiovascular, metabolic or hormonal co-morbidity and mortality. Low social status or income, unemployment, life events such as divorce, negative lifestyle habits, and professional requirements (e.g., shift work) are often associated with sleep problems. Sleep disorders affect the quality of life and impair both professional and non-professional activities. Excessive daytime drowsiness resulting from sleep disorders impairs efficiency and safety at work or on the road, and increases the risk of accidents. Poor sleep (either professional or voluntary) has detrimental effects comparable to those of major sleep disorders, but is often neglected. The high incidence and direct/indirect healthcare and welfare costs of sleep disorders and poor sleep currently constitute a major medical problem. Investigation, monitoring and strategies are needed in order to prevent/reduce the effects of these disorders.

https://www.mdpi.com/1660-4601/13/8/831/pdf

Co-Morbidity, Mortality, Quality of Life and the Healthcare/Welfare/Social Costs of Disordered Sleep: A Rapid Review

Study Objectives:Describe common symptoms, comorbidities, functional limitations, and treatment responsiveness among patients with narcolepsy. Investigate the effect of pediatric onset of narcoleps...

Listening to the Patient Voice in Narcolepsy: Diagnostic Delay, Disease Burden, and Treatment Efficacy.

The clinical spectrum of childhood narcolepsy.

Attention deficit/hyperactivity disorder (ADHD) is one of the most commonly diagnosed disorders in childhood, enduring through adolescence and adulthood and presenting with symptoms of inattention, hyperactivity, and/or impulsivity and significantly impairing functioning. Primary sleep disorders such as sleep-disordered breathing, restless leg syndrome, circadian rhythm sleep disorder, insomnia, and narcolepsy are commonly comorbid in these individuals but not often assessed and are therefore often left untreated. Sleep disturbances in individuals with ADHD can result in significant functional impairments that affect mood, attention, behavior, and ultimately school/work performance and quality of life. Previous reviews have described findings related to sleep but have neglected to examine potential impacts of these sleep disorders and ADHD on daytime functioning. This review investigates empirical findings pertaining to sleep abnormalities and related cognitive, behavioral, emotional, and physical impairments in individuals with ADHD and comorbid primary sleep disorders across the life span. It discusses implications to management and highlights existing limitations and recommended future directions.

https://www.dovepress.com/getfile.php?fileID=46986

Katharina Hingerl

Papers

Subjective deficits of attention, cognition and depression in patients with narcolepsy.

Authors response to “Deficits of attention and cognition in narcoleptic patients – is it hypocretin dependent?”